Help please.

[luggsey]

Hi all, new here I'm sad to say... Mum is in hospital with vasculitus, symptoms started off with pneumonia which she had antibiotics for, went downhill over days from there, loss of feeling in fingers, hard to walk, double vision, no interest in anything, collapse, suspected stroke, icu, stroke ward, kidney failure, icu again, transfer to large area hospital for dialysis, finally had a positive anca test. Kidney biopsy shows vasculitus but type not certain. Treated with perdistone and cyclophosmide which seems to work. Steroids dose at *I think 40mg and cyclophosmide at 100mg. Mums 70 and 67kg. Her collapse was in mid march and she is now in a rehab ward trying to get on her feet. Eating ok now, kidneys restarted after weeks on dialysis, thank god for that. Question, what to expect next? she has a three week apointment to see a Renal consultant that I presume deals with vasculitus as well. What should I be doing and asking to help her? I am terrified a common infection will overwhelm her, when will she be ok? I have been off work with stress and grief for weeks now and need some help please.

[Ayla Volunteer]

Hello Lugsey!

Sorry to read about the stress and worry you are going through and, of course, about the experiences your mother has suffered. I know a lot of us on this site will be nodding, recognising from what you say the rocky road to diagnosis and the 'medical sledgehammer' experience of the medication your mum is receiving.

From what you say, your mum is on the correct drugs, and at the correct doses (these are determined by the patient's weight). It is likely that the dose of the steroid will be gradually reduced, presuming your mother's next lot of blood tests are ok. To quote my own experience, I started on 60mg daily, reduced to 45mg at the next appointment, then down to 30mg at the next, and 20mg a few months later. The reducing regime will slow then, it may take a year or more to get down to around 5mg. It has taken me two! It will all depend on how your mum responds to treatment.

The cyclophosphamide is different. It is an immune system suppressant, I suspect you know that. It is normally given for 3-6 months at the starting dose, until a chemically induced remission is obtained. Blood tests will, again, show when this happens. After this, a milder drug is substituted, normally azathioprine or something similar. Just be aware, at the high dose start of treatment, your mum will be feeling very tired indeed! This is normal. She will be on her way to feeling much better, though, as the disease is brought under control and, when this happens, the drugs are reduced.

I would advise you ask your mum to make a list of every symptom she has and to take this list with her to her next appointment. Everything, however unconnected it seems, is important in giving her consultant a full picture of how she is and how his prescribed therapy is working. If rashes, ulcers, red eyes or swellings, etc. occur, then take photographs and date them. All useful! Also, once she is home, don't hesitate to ring thr renal consultant's secretary and ask to speak to the consultant, if you are worried about anything concerning your mum's symptoms.

Infection risk? Well, it is certainly there. Just avoid anyone with a cold or other illness, wipe down touchable parts of the home with anti-bac stuff regularly, eat well, etc and keep your fingers crossed! If your mum does go down with something, let the GP know immediately as she will need antibiotic treatment sooner rather than later.

It would be good if you and your mum could read a lot about vasculitis. If you can access the Vasculitis UK website, that contains a lot of helpful information and advice, especially the Route Map which is downloadable.

Just one question - what type of ANCA result did your mum have? It will be either C ANCA or P ANCA. That will help determine which type of vasculitis she is suffering from.

Try not to worry, just aim to ask a lot of questions and thus learn a lot. And keep an eye on how your mum is feeling, this is a good indicator of how things are going. I am hoping that right now is the darkest hour just before a better dawn for you.

Ayla

[Ayla Volunteer]

vasculitis.org.uk/about/rou...

This is the hyperlink to the Vasculitis UK website (Route Map page).

Ayla

[Jann]

Oh what a rough time your mother has had and it is hard for you too. My daughter and son where very upsetting when I was taken into hospital and I was not as badly off as your mother sounds she was when taken to hospital. I too was ANAC positive. The symptoms your describe your mother as having are common to vasculitis suffers. You do not say how long you Mum's cyclophosmide treatment was for. A typical regime might see your mother have three to six months’ worth of infusions. They are normal about three weeks apart but can vary for a number of reasons. During this period the steroid dose may be reduced slowly if a patient is looking like they are responding to the overall treatment. At three or six months the consultant is likely to change to another drug. A list of the possible drugs can be found at vasculitis.org.uk/about-vas... the same drugs are used for MPA suffers.

She will need to see a consultant regularly about every 8 -12weeks for blood tests. They check your kidney function and your ANAC results among other things.

As your mother has kidney damage her primary consultant may be a renal consultant.

I did not have to learn to walk again but a friend of mine did and he is about your mother’s age and is now back on his feet. However this is not an area I know anything about.

If you look at the Vasculitis UK web site you may find more information that may help.

One think your Mum will not want is you getting sick with worry. It is an easy thing to say and very hard to do. Keep talking to people on this site and F/B and you will learn more about you’re your mother condition and hopefully you will both learn to living with it.

Best wishes to you both.

[John_Mills Volunteer]

I can't really add anything to Ayla's excellent & comprehensive reply except to say that vasculitis is usually treated by either renal or rheumatology consultants. The important thing now is the extent to which the kidneys have recovered. Anything above 50% function is good, although it may take time for the full potential for recovery to take place.

Once the disease is controlled and maintenance takes place, the future can be very good. Do make sure Mum gets all the recommended vaccinations to protect her.

John

[PatriciaAnn]

Hi Luggsey

WE are sorry that that you have need to find us, but at least you've come to the right place. None of us are doctors we are just patients who have vasculitis of one sort or another. We try to offer help based on what we have been through and what we have picked up along the way.

The treatment your mum is on is very much in line with what most of us have had. Prednisolone is a steroid to get the inflammation down, the cyclophosphamide is a low dose chemotherapy to zap her immune system because what is happening is her immune system is not protecting her but is fighting her. So zap the system and it can't attack itself. (Simplified answer).

What to expect, well that isn't something for which there is a simple "one size fits all" answer. Vasculitis diseases are nasty little ........ers and it all depends on which vasculitis, which organs are affected and what damage has been done before we are diagnosed and start treatment. The treatment itself is pretty ropey.

Added infection is something to be aware off. If your immune system is zapped then it can't protect you from infection, so a great deal of care must be given to good hygiene and trying to keep away from anyone with colds and other problems. Sometimes easier said than done.

When it is Mum's appointment then go with her. He/she (consultant) will probably tell you what the medical plan is for Mum. It is difficult to know what to say you ought to ask for this first visit. Best listen and hopefully all the points will be covered. One of the things you will have to come to terms with is that most of the vasculitis diseases cannot be cured but many of us are in remission (even if it is drug induced remission). Unfortunately vasculitis can be fatal if not diagnosed quickly and treated correctly.

I do so hope your Mum is going to be OK. Sounds as if the doctors treating her know what they are about.

Finally, Vasculitis UK have a very good website and everything there has been written by or verified by medical professionals who are experts in the vasculitis field. Please be careful what you read on the internet. Lots of it is out of date and lots of it just isn't correct.

Long reply, hope you didn't fall to sleep half way through.

PatriciaAnn

[luggsey]

Thank you for your replies, just got back from seeing mum and thankfully she seems to be doing well. She tires very quickly even when hoisted out into a physio chair but can now stay out in a chair for several hours. Good movement is returning to her arms and legs, strength in the neck now, still has weak trunk and legs plus pins and needle hands. As I explain to all the medical professionals she had these symptoms pre stroke so I assume its the vasculitus that has done this by affecting the tiny blood vessles feeding the nerves. I don't know how long its going to take for the.feeling to fully return but it has started.

Her kidneys have 'normal' function now, there was no scar tissue on her kidney biopsy but the type of vasculitus is unclear, its not the sort they expected...The consultant said it fell between two different types? My mum is an extremely nervous person and frightened of medical procedures so they didn't try any more biopsys or MRI or xray with dye (can't remember the name) because the treatment would be the same anyway.

Her steroid dose is down to 25mg and will drop again to 20mg shortly. I presume this means she is responding well? Is this drugs dose worked out on blood test inflamatory markers? I don't know how the cyclophosmide dose is adjusted, is this based on anca tests? I'm hoping for her to be on a 'safer' dose of drugs soon, how compromised is her immune system when on 100mg oral daily dose? I have told her to drink lots throughout the day to help protect her bladder, nobody else has told her this? In fact nobody tells us anything unless we ask! So how long will her immune system be badly compromised? This seems to be my biggest concern, does the blood test full count reveal the condition of the immune system? What's the chance of infection being serious, has that happened to anybody here?

Sorry to rattle on, just that I'm so scared of this.

[Ayla Volunteer]

rheumatology.org/Practice/C...

Try this weblink, Luggsy, it will answer a lot of your questions about cyclophosphamide. You are right about getting her to drink extra water, this will help to flush out her system and protect her bladder. It is, I believe, normal to stay on her present dose of cyclophophamide until remission is achieved, unless the liver is adversely affected en route, in which case the cyclo will be stopped and another drug substituted. This happened to me after 2 months of treatment. Luckily, I was by then in remission and could move on to a less toxic immunosuppressant but, had I not been, there are other options.

Your mum's doctors will be working from her blood test results and you can rest assured they would not be reducing the steroid dose unless they were satisfied that she was responding well to treatment. I am pleased to hear that her kidneys have recovered normal function, that is excellent news,

In everyone with an auto immune disease, which includes all vasculitis patients, the immune system attacks its own body cells in the mistaken belief that they are foreign invaders. It is to reduce the power of the immune system that your mother has been given cyclophosphamide, the immunosuppressant drug. The steroid (prednisolone) reduces inflammation and I believe that it, too, weakens the immune system. The degree to which the immune system is impaired is predictable and linked to the dosage of both drugs. Vasculitis patients have an amount given that is determined by body weight and your mother is being given the correct amount of cyclophosphamide. Her immune system will still have some power left to fight infection but it will be considerably reduced. This may well continue for the rest of her life, though she will get used to avoiding sources of infection and life does return to near normal, all being well.

It is possible to temporarily halt the immunosuppressant drug, if any future infection becomes serious. Perhaps that is reassuring?

I hope this helps. Keep asking the questions and good luck to you both!

Ayla

[luggsey in reply to Ayla]

Thanks for this, its the infection risk that scares me, I wonder how soon after stopping cyclophosmide the immune system returns to normal?

My mum is prone to chest infections and had pneumonia twice recently.

Has anyone heard of anyone having serious problems with infections?

[Ayla Volunteer]

No, I haven't, but that doesn't mean it hasn't happened! Speaking for myself, I get the occasional cold which never got serious pre the drugs I am on but now usually goes onto my chest and needs at least one course of antibiotics. Recently I had a bad dose of food poisoning which refused to clear (despite the GP telling me it would within 3 - 5 days!) and my immunosuppressant was stopped for 10 days, on the advice of the consultant, to allow my body to tackle it. I finally recovered after 11 days.

There is always the risk of infection turning more serious than it normally would when you are on drugs that suppress your immune system. The only comfort is that your mum will be closely monitored and, providing she reports any sign of infection straight away, it will be dealt with before it can get a serious hold.

To answer your other question about how soon the immune system returns to normal, I really don't know the answer. I was told that the drugs we take stay in your system for about 9 days after you stop taking them but, as most of us with vasculitis have never been drug free, we haven't had the opportunity to test this out!

Try not to worry too much.

Ayla

[AndrewT]

Dear luggsey,

Sorry to hear about your mum, give her my-our- love will you please. I can't add much, to what has been said above, except try not to worry too much; hard I know. My vasculitis affected my kidneys and I am now on dialysis, have been for about three years now, so I know all about that too!

If I can be any more help please let me know.

Best wishes

AndrewT

[luggsey]

I don't know that I can stop the worrying, knowing more about the condition is important to me so I can help my mum as much as I can. She is in a community hospital now and to be honest they are clueless as to her condition. Knowing just that bit more helps me to help her.

I took her out in a wheel chair for half an hour today, she loved the sun on her face and hearing the birds singing. I pray she will recover to enjoy her life, she is only seventy and had been fit and well until this awful illness.

Thanks for all the help and kind comments.

[luggsey]

Mum has been told her inflammation has dropped, this must be good news? Does this mean the perdistone dose will be dropped again? He drug chart says drop to 20mg pending 'Renal review' which I don't really understand as her kidneys are working normally?

She has also been told her sodium level is low, but I don't know what action they take about that? The strange thing is her protein level is low, she is eating ok so is this caused by drug side effects? They are sending the dietition to speak to her?

Her appointment with the Renal consultant is just under two weeks away. I know I need to ask what sort of vasculitus she has, how long she will be taking perdistone and cyclophosmide?

[luggsey]

Confusing information from the hospital, now the CRP is slightly up to 77, Albumin 29, urea 11.7, do these values sound like a flare or are they to small?